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Updated: Oct 25, 2025
A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Surgical Practice in Girls with Congenital Adrenal Hyperplasia: An International Registry Study
Doris Hebenstreit1, S Faisal Ahmed2, Nils Krone3
1Department of Urology, Hanusch Krankenhaus, Vienna, Austria.
Insights
Surgical practices for girls with congenital adrenal hyperplasia (CAH) vary globally. This study reveals a trend towards vaginal surgery within two years, particularly in Asia and South America, while Europe reports fewer interventions.
Area of Science:
- Endocrinology
- Pediatric Surgery
- Genetics
Background:
- Congenital adrenal hyperplasia (CAH) is a group of genetic disorders affecting the adrenal glands.
- Surgical interventions for CAH, particularly genitoplasty, are a subject of ongoing debate.
- International trends in surgical management for 46,XX CAH have not been comprehensively evaluated.
Purpose of the Study:
- To evaluate international trends in surgical practices for girls with 46,XX congenital adrenal hyperplasia (CAH).
- To identify geographical and temporal variations in surgical decision-making for CAH.
- To analyze the trends in early surgical interventions for CAH.
Main Methods:
- Retrospective analysis of cases classified as 46,XX CAH in the I-CAH/I-DSD registry, born before 2017.
- Inclusion of 330 participants, with data collected from participating centers regarding surgical decisions.
- Logistic regression modeling was used to assess the probability of early surgery over time.
Main Results:
- Genital surgery was performed in 75.8% of the 330 participants.
- Surgical rates varied significantly by region: 64.3% in Europe, 89.2% in South America, and 96.8% in Asia.
- A trend towards increased probability of early vaginal surgery (within 2 years) was observed over time, but not for clitoral surgery or combined procedures.
Conclusions:
- Genitoplasty in girls with CAH remains a controversial practice with significant international disparities.
- The study highlights geographical and temporal differences in surgical approaches to CAH.
- There is an emerging trend favoring early vaginal surgery within the first two years of life, particularly in Asian and South American centers.
Abstract:
In this article international trends in surgical practice in girls with congenital adrenal hyperplasia (CAH) are evaluated. All cases that had been classified in the I-CAH/I-DSD registry as 46,XX CAH and who were born prior to 2017 were identified. Centers were approached to obtain information on surgical decision making. Of the 330 included participants, 208 (63.0%) presented within the first month of life, and 326 (98.8%) cases were assigned female. Genital surgery had been performed in 250 (75.8%). A total of 64.3, 89.2, and 96.8% of cases residing in Europe, South America and Asia, respectively, had at least one surgery. In a logistic regression model for the probability of surgery before the second birthday (early surgery) over time an increase of probability for early vaginal surgery could be identified, but not for clitoral surgery or both surgeries combined. Genitoplasty in girls with CAH remains controversial. This large international study provides a snapshot of current practice and reveals geographical and temporal differences. Fewer surgeries were reported for Europe, and there seems to be a significant trend towards aiming for vaginal surgery within the first 2 years of life.
